Ten step approach to movement disorders

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Ten step approach to movement disorders

  1. 1. TEN STEP APPROACH TO MOVEMENT DISORDERS Prof. A.V. SRINIVASAN, MD, DM, Ph.D, F.A.A.N, F.I.A.N, EMERITUS PROFESSOR TAMILNADU DR.M.G.R MEDICAL UNIVERSITY CHENNAI FORMER PROFESSOR AND HEAD INSTITUTE OF NEUROLOGY MADRAS MEDICAL COLLEGE12/06/2010 Diamond jubliee year(1950-2010) 1
  2. 2. MOVEMENT DISORDERS• Step 1 What are the Movements ?• Step 2 Identify the overall syndrome• Step 3 Decide the disease/Syndrome pattern from differential diagnosis• Step 4 If not, is it Odd dyskinesias?• Step 5 Emphasis on clinical clues and diagnostic pathway• Step 6 If primary movement disorder – Principle investigations• Step 7 General Plan• Step 8 Investigations for Symptomatic Movement Disorders• Step 9 Additional tests in specific clinical syndromes• Step 10 Guidelines for Movement Disorders in children/Young Adults 12/06/2010 Diamond jubliee year(1950-2010) 2
  3. 3. STEP 1 – WHAT ARE THE MOVEMENTS 1. AKINETIC OR DYSKINETIC TREMOR JERKS Myclonus Chorea Tic SPASMS Dystonia Rhythmic / arhythmic Stereo typed / in consistant Continous Action Paroxysms12/06/2010 Diamond jubliee year(1950-2010) 3
  4. 4. STEP 2 – IDENTIFY WHAT IS THEOVERALL SYNDROME• Akinetic rigid syndrome Dystonic syndrome Choreic syndrome Tic syndrome Myoclonic syndrome12/06/2010 Diamond jubliee year(1950-2010) 4
  5. 5. STEP 3 – WHAT IS THE CAUSE ?• Differential diagnosis of various syndrome• See standard- text book12/06/2010 Diamond jubliee year(1950-2010) 5
  6. 6. STEP 4 – ODD DYSKINESIASA. ODDTREMOR Mid brain tremor Task specific tremor Neuro pathic tremor Dystonic tremor Primary orhtostatic tremor12/06/2010 Diamond jubliee year(1950-2010) 6
  7. 7. STEP 4 – ODD DYSKINESIASB. ODD JERKS1.FOCAL MYOCLONUS• Angio endothelioma- s1- root Toe jerks alone2.CORTICAL MYOCLONUS• Encephalitis Jerks of posture Action myoclonus Stimulus sensitive myoclonus12/06/2010 Diamond jubliee year(1950-2010) 7
  8. 8. STEP 4 – ODD DYSKINESIAS• B. ODD JERKS3. GIANT SOMATO SENSORY• Syrinx• Repetitive jerks lower limbs4. HYPEREXPLEXIA5. ODD SPASMS• PLMT• Hemidystonia12/06/2010 Diamond jubliee year(1950-2010) 8
  9. 9. STEP – 5 EMPHASIS ON CLINICAL CLUES AND DIAGNOSTIC PATHWAYEncephalopathy and lowdensity lesions No infection – Urea cycle defect mitochonrdial orin MRI pyruvate disorder, organic acid disorderOrganomegaly Wilson’s Gaucher’s Niemann Pick disease GalactosaemiaPeripheral Neuropathy Adreno myelo – leucodystrophy GM2 Gangliosidosis Krabbe’s disease Meta Chromatic leukodystrophy Gaucher’s disease Mucolipidosis Mitochondrial disordersMyoclonus and epilepsy Lafora body disease ceroid lipo fuscinosis GM2 Gangliosidosis Gaucher’s disease Polychstic lipomembranous asteodysplasia Mitochondrial disease. 12/06/2010 Diamond jubliee year(1950-2010) 9
  10. 10. STEP – 5EMPHASIS ON CLINICAL CLUES ANDDIAGNOSTIC PATHWAY Macrocephaly Alexander’s disease metachromatic leukodystrophy Muscle weakness and wasting Neuronal Intranuclear inclusion disease Vertical supra Nuclear Palsy Niemann pick disease Gaucher’s Disease Cherry Red spotin Macula Sialidosis GM & GM2 gangliosidosis Memann Pick’s disease Dysmorphic features Mucopolysacridoses Mucolipodiosis Investigations for primary movement disorder12/06/2010 Diamond jubliee year(1950-2010) 10
  11. 11. STEP – 6INVESTIGATIONS FOR PRIMARYMOVEMENT DISORDERS Imaging (MRI) Exclusion of Wilson <50) Genentic testing Routine blood wing Biochemistry Syphilis12/06/2010 Diamond jubliee year(1950-2010) 11
  12. 12. STEP – 7 GENERAL PLAN Extent of nervous system involvement Psychometric evaluation EEG (epilepti form discharges) ENMG (peripheral neruropathy) EMG and VEP12/06/2010 Diamond jubliee year(1950-2010) 12
  13. 13. STEP – 8INVESTIGATIONS IN SYMPTOMATICMOVEMENTDISORDERS METABOLIC AND STORAGEDISORDERS• Metabolic encephalopathies categories and investigation• Metabolic Storage Disorders: Categories And Investigation• Degenerative And Systemic Disorders12/06/2010 Diamond jubliee year(1950-2010) 13
  14. 14. STEP 9 : ADDITIONAL TEST TOSPECIFIC CLINICAL SYNDROMS Smptomatic parkinsonism MSA (Anal or uretheral EMG) MRI – Low density in GB/Putamen MSA / PSPSYMPTOMAIC TREMORS• T3T4 – Thyrotoxicosis• Peripheral Neuropathy Paraprotenemias• Hg. Poisoning• Unilateral tremors – opp. Basal ganglia, Thalamus, Sub Thalamic body of Luys.12/06/2010 Diamond jubliee year(1950-2010) 14
  15. 15. STEP 9 : ADDITIONAL TEST TOSPECIFIC CLINICAL SYNDROMSSYMPTO, CHOREA• Neuroacanthocystosis – peripheral smear /CK T3,T4 – Thyrotoxicosis Polycythemia rubravira Calcium and magnesium metabolism Hyponatremia Auto immune disorders Syden ham’s chorea SLE APLS Struct, lesion of Sub Thalamic Body of luy.12/06/2010 Diamond jubliee year(1950-2010) 15
  16. 16. STEP 9 : ADDITIONAL TEST TOSPECIFIC CLINICAL SYNDROMSSYMPTOMATIC TIC – NeurocanthocytosisSYMPOTOMATIC MYOCLONUS• Establish the site of origin n the nervous system by electrophysiology Lafora body disease Neuronal ceroid lipofuscinosis Sialidosis Mitochondrial disorders• Unverricht Lundborg Disease12/06/2010 Diamond jubliee year(1950-2010) 16
  17. 17. STEP 9 : ADDITIONAL TEST TOSPECIFIC CLINICAL SYNDROMSSYMPT. DYSTONIA (RARE) Niemann Pick type C – Bone marow Sea blue histiocytes DRD Sandifer syndrome Atalanto axial subluxation (fixed painful torticollis)SYNDROME WITH CONTINOUS MUSCLE FIBRE ACTIVITY• Detailed ENMG study Episodic or paroxysmal movement disorders Video telemetry EEG / distinquish from epilepsy Paroxysmal spasm – M.S. Intermitant ataxias – Amino acid disorders12/06/2010 Diamond jubliee year(1950-2010) 17
  18. 18. STEP 9 : ADDITIONAL TEST TOSPECIFIC CLINICAL SYNDROMSINVASIVE INVESTIGATIONS• Skin biopsy (Axilla) Muscle biopsy Peripheral nerve biopsy• Brain biopsy12/06/2010 Diamond jubliee year(1950-2010) 18
  19. 19. STEP – 10 :GUIDE LINES FRO MOVEMNET DISORDERS INCHILDREN /YOUNG ADULTS• CHILDHOOD NEURODEGENERATIVE DISEASES THAT MAY PRESENT IN YOUNG ADULT LIFE WITH A MOVEMENT DISORDER• SPECIAL STUDIES TO BE CONSIDERED IN CHILDREN OR YOUN ADULTS WITH A SYMPTOMATIC MOVEMENT DISORDER12/06/2010 Diamond jubliee year(1950-2010) 19
  20. 20. MYOCLONUS IN THE SETTING OFEPILEPSY• Generalized Idiopathic Epilepsy• Progressive Myoclonic Epilepsy• Neuronal Ceroid Lipofuscinosis• Mitochondrial Encepalomyopathy• Sialidosis• Lafora Body Disease12/06/2010 Diamond jubliee year(1950-2010) 20
  21. 21. MYOCLONUS IN THE SETTING OFEPILEPSY• Unverricht-Lundborg Disease:”Baltic Myoclonus”• Miscellaneous Causes of PME• Action Myoclonus• Postthpoxic Myoclonus• Focal Myoclonus• Palatal Myoclonus• Epilepsy Partialis Continua• Segmental Myoclonus.12/06/2010 Diamond jubliee year(1950-2010) 21
  22. 22. STARTLE EPILEPSY• UNILATERAL STARTLE - HEMITONIC SPASM - HEMI ATONIC ATTACK• BILATERAL STARTLE - GENERALISED TONIC SPASM• GLOBAL ATONIC ATTACK12/06/2010 Diamond jubliee year(1950-2010) 22
  23. 23. TREATMENT OF STARTLE EPILEPSY• Clobazam• Clonazepam• Carbamazepine• Chlordiazepoxide12/06/2010 Diamond jubliee year(1950-2010) 23
  24. 24. ACQUIRED PAROXYSMALDYSKINESIAS• Multiple sclerosis• Myelopathy• Vascular or developmental anomalies• Drug-induced paroxymal dyskinesias• Cerebral ischemia• Head trauma• Cerebral palsy• Hemiparasis• Focal seizures12/06/2010 Diamond jubliee year(1950-2010) 24
  25. 25. ACQUIRED PAROXYSMALDYSKINESIAS• Encephalitis• Radiculapathy• Hypoparathyroidism• Thyrotoxicosis• Hypoglycemia• Psychogenic• Reflex sympathetic dystrophy.12/06/2010 Diamond jubliee year(1950-2010) 25
  26. 26. Seizures & paraxymal dyskinesias• CPS – Temporal lobe• Two cases- movement themselves to be epileptic• In another two cases- paraxymal dyskinesias blended with GTCS12/06/2010 Diamond jubliee year(1950-2010) 26
  27. 27. KOTAGAL- TEMPORAL LOBE• Unilateral dystonic posturing with super imposed choreo-athetosis• Other anotomical slides• 1. mesial frontal,• 2. frontal sagittal,• 3. fronto temperal.12/06/2010 Diamond jubliee year(1950-2010) 27
  28. 28. EPILEPSY & MOVEMENT DISORDERS –OTHER CONDITIONS 1. REM Sleep behavior disorder 2. PLMS 3. RETT syndrome 4. Drug induced movement disorder 5. Frontal lobe automatism12/06/2010 Diamond jubliee year(1950-2010) 28
  29. 29. REM SLEEP BEHAVIOURDISORDER• Idiopathic• Secondary12/06/2010 Diamond jubliee year(1950-2010) 29
  30. 30. PLMS12/06/2010 Diamond jubliee year(1950-2010) 30
  31. 31. RETT SYNDROME• Infantile spasm• Myocolonic seizures• Abnormal early development12/06/2010 Diamond jubliee year(1950-2010) 31
  32. 32. DRUG-INDUCED MOVENTDISORDER• Valproic acid – parkinsonism, chorea, oculogyris chyris• Phenytoin – chorea achetosis• Anti-psychotic drugs – dystonia12/06/2010 Diamond jubliee year(1950-2010) 32
  33. 33. FRONTAL LOBE AUTOMATISM 1. Oroalimentary(lip smacking, chewing, swallowing, licking) 2. facial (distorted facial expressions, frequently giving the appearance of fear) 3. Upper extremity (fumbling, clasping, grabbing movements of the hands: repetitive touching, sxratching, or rubbing of objects)12/06/2010 Diamond jubliee year(1950-2010) 33
  34. 34. FRONTAL LOBE AUTOMATISM 4. Lower extremity-ambulatory (walking, runnig, bicycling movements of the legs, kicking). 5. Sexual (pelvic thrusting, masturbation). 6. Vocalization (sounds, words, or phrases).12/06/2010 Diamond jubliee year(1950-2010) 34
  35. 35. CONCLUSION Epileptic seizures presenting as motor phenomena without concomitant conscious change may be confused with one the paroxymal movement disorders. Conversely, the attack of paroxymal movement disorders maybe thought to be epileptic due to a number of factors, including its sudden, unpredictable, and transient nature, its responsie to anti convulsants, and the premonitary sensations preceding attacks. The distinction between epilepsy and movement disorders is further confused by the reports that these two conditions frequently occur in the same families or even in the same patients. Recent-- (PTO)12/06/2010 Diamond jubliee year(1950-2010) 35
  36. 36. CONCLUSION --studies shows that a few epilepsy and paroxymal movement disorders are “channelopathies” , indicating that they may share some common pathophysiology and a possible “over lap”. A good quality of history, a trail to reproduce the motor phnomena, the application of video-EEG, polysomnography, and other electrophysiological recordings, together with regular follow-up are important for differentiating these two conditions.12/06/2010 Diamond jubliee year(1950-2010) 36

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